Abstract
Rendu-Osler-Weber disease is a rare autosomal dominant disorder. Hepatic involvement manifests itself as vascular, parenchymal, and biliary lesions with characteristic telangiectasias and vascular shunts. In a 37-year-old female patient, dynamic contrast-enhanced upper abdominal CT and MRI were performed. CT and MRI revealed dilated celiac trunk and hepatic artery. On early arterial phase, dilated hepatic veins showed significant enhancement. On arterial and portal venous phases, liver showed significantly heterogeneous contrast enhancement and showed homogenous enhancement in the hepatic parenchymal phase. On the magnetic resonance cholangiopancreatography, irregular biliary ducts with strictures and dilatation were seen.
Highlights
Hereditary haemorrhagic telangiectasia (HHT), known as Rendu-Osler-Weber disease, is a very rare hereditary autosomal dominant vascular disorder that occurs with an estimated frequency of 1–20 cases/100,000 [1]
Opacification of the hepatic veins during the arterial phase was considered as an indirect sign of the presence of hepatic-arteriosystemic venous shunt
And prolonged enhancement of the portal vein during the arterial phase was considered an indirect sign of the presence of arterioportal shunt
Summary
Hereditary haemorrhagic telangiectasia (HHT), known as Rendu-Osler-Weber disease, is a very rare hereditary autosomal dominant vascular disorder that occurs with an estimated frequency of 1–20 cases/100,000 [1]. Hepatic involvement occurs in up to 31% of cases and consists of vascular, parenchymal, and biliary lesions with characteristic telangiectasias and vascular shunts [2]. The gold standard for diagnosing liver involvement in HHT is selective digital subtraction angiography (DSA) of the hepatic artery; it is an invasive method for the screening and diagnosis. Computerized tomography (CT) and magnetic resonance imaging (MRI) play a central role in the visualization of hepatic changes [4]. There were few reports about the value of MRI in this disease, despite the fact that this technique allows imaging of the liver parenchyma, biliary tract, and hepatic vessels. Magnetic resonance cholangiopancreatography (MRCP) is a noninvasive technique in the detection and characterization of bile duct abnormalities [2]
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